Kaposi Sarcoma in the Setting of Cushing Disease

ObjectiveTo report a case of a human immunodeficiency virus (HIV)-negative Kaposi sarcoma (KS) associated with Cushing disease (CD).MethodsThe details of case presentation, evaluation, diagnosis, and treatment are presented and cases of KS and CD published before November 1, 2010 on PubMed and Scopus are reviewed.ResultsA 54-year-old Hispanic HIV-negative man presented with typical signs and symptoms of CD (easy bruisability, proximal muscle wasting, and abdominal fat pads). Numerous raised, purplish, nonblanching plaques 0.5 to 2 cm in diameter extended throughout his lower extremities. Biochemical tests and pituitary magnetic resonance imaging confirmed CD. A lesion biopsy showed atypical vascular proliferation positive by immunohistochemistry for human herpesvirus 8 (HHV-8), consistent with KS. He underwent 2 transsphenoidal surgeries followed by a bilateral adrenalectomy. After recovery, his KS was treated with a systemic combination of liposomal doxorubicin and paclitaxel.ConclusionThe occurrence of CD and KS is rare. Specific therapy for CD and chemotherapy for KS are effective in the treatment of KS associated with CD.